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Transcript
Hydrolysis of membrane-associated ceramide by acid ceramidase in the presence of saposins and bis(monoacylglycero)phosphate (BMP) in the acidic lysosomal compartment. According to a recent model on the topology of lysosomal digestion,47 ceramide hydrolysis takes place on intralysosomal vesicles. R = (CH2)7CH=CH(CH2)7CH3. Source: Acid Ceramidase Deficiency: Farber Lipogranulomatosis, The Online Metabolic and Molecular Bases of Inherited Disease Citation: Valle D, Beaudet AL, Vogelstein B, Kinzler KW, Antonarakis SE, Ballabio A, Gibson K, Mitchell G. The Online Metabolic and Molecular Bases of Inherited Disease; 2014 Available at: http://mhmedical.com/ Accessed: August 03, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved
